Isolated ventricular apical hypoplasia: A report of four cases and literature review.

Isolated ventricular apical hypoplasia (IVAH) is a rare congenital cardiac anomaly, with clinical manifestations depending on the age of the patient, ranging from no symptoms in children to congestive heart failure or even malignant tachycardia in adults. Herein, we describe the clinical and anatomical findings in four cases with hypoplasia of the right or left ventricular apex, and we discuss the possible mechanisms and differential diagnosis of this malformation. Echocardiography is a rapidly accessible, low cost, noninvasive technique for the detection and evaluation of IVAH.

Knockout of Toll-like receptor 4 improves survival and cardiac function in a murine model of severe sepsis.

Toll-like receptor 4 (TLR4) is a transmembrane pattern­recognition receptor expressed in immune cells and the heart. Activation of TLR4 signaling during sepsis results in the release of cardiac depression mediators that may impair heart function. The present study aimed to determine whether TLR4 contributes to development of severe sepsis­induced myocardial dysfunction. A cecum ligation and puncture (CLP) procedure was employed to establish severe sepsis models. Wild type (WT) and TLR4 knock­out (TLR4­KO) mice were divided into four groups: WT­sham, TLR4­KO­sham, WT­CLP, and TLR4­KO­CLP. Cardiac function of these animals was evaluated at various time points following the surgical procedure. The expression levels of proinflammatory cytokines in the heart tissues were detected by reverse transcription­semi quantitative polymerase chain reaction (RT­PCR). Myocardial neutrophil and macrophage infiltration were investigated by histopathological examination, as well as a myeloperoxidase activity assay in heart tissue by RT­PCR. Myocardium Fas cell surface death receptor/Fas ligand and caspase­3 were also analyzed by RT­PCR. Additionally, myeloid differentiation primary response 88 M, toll or interleukin­1 receptor­domain­containing adapter­inducing interferon­ß and nuclear factor­κB expression levels were observed in the myocardium of all four groups. WT­CLP mice exhibited increased mortality rates, more severe cardiac dysfunction and had increased levels of interleukin (IL)­1ß, IL­6 and tumor necrosis factor­α in heart tissues and increased neutrophil infiltration compared with TRL4­KO­CLP mice. The present study reported that TLR4 aggravates severe sepsis­induced cardiac impairment by promoting the release of proinflammatory cytokines and neutrophil infiltration in hearts.

The inhibition of MyD88 and TRIF signaling serve equivalent roles in attenuating myocardial deterioration due to acute severe inflammation.

Myeloid differentiation factor 88 (MyD88) and Toll or interleukin-1 receptor-domain-containing adaptor-inducing interferon-ß (IFN-ß) (TRIF) are two pivotal downstream adaptors of Toll-like receptors. Activation of MyD88 or TRIF signaling in cardiac immune pathology of severe inflammation negatively influences heart function. In the present study, severe septic cardiac injury was induced in C57BL/6 mice by cecum ligation and puncture (CLP). A total of 64 mice were divided randomly into the following four groups (n=16/group; 8 for observation of survival rate, 8 for heart sample analysis): Sham, CLP, anti-MyD88-CLP and anti-TRIF-CLP. Anti-MyD88 and anti-TRIF antibodies were administered to the respective mice through the tail veins 2 h before CLP. Measurements of cardiac function, including M-modes, velocity vector imaging and cardiac troponin I, were performed. Myocardial inflammatory cytokines were examined by reverse transcription-polymerase chain reaction (RT-PCR), myocardial neutrophil infiltration was measured by a myeloperoxidase activity assay, intracellular adhesion molecule and vascular cell adhesion molecule mRNA expression levels were investigated, and histopathological characteristics were evaluated. Levels of mRNA transcripts encoding genes for apoptosis production and MyD88, TRIF, nuclear factor-κB and IFN regulatory factor 3 were investigated by RT-PCR. Mice challenged with CLP demonstrated deleterious cardiac function, increased levels of interleukin-1ß (IL-1ß), IL-6ß, and tumor necrosis factor-α mRNA, increased neutrophil infiltration, and increased apoptosis. In contrast, mice in the anti-MyD88 CLP and anti-TRIF CLP groups retained cardiac function with reduced cytokine release, decreased neutrophil infiltration, and reduced apoptosis. In addition, there was no significant difference between the anti-MyD88 CLP and anti-TRIF CLP groups. Thus, the present study indicated that MyD88 and TRIF blockades serve notable and equivalent roles in protecting cardiac deterioration from severe sepsis by attenuating cytokine release, reducing neutrophil infiltration and alleviating apoptosis.

Isolated right ventricular apical hypoplasia characterized by computed tomography and echocardiography.

Isolated right ventricular apical hypoplasia is an unusual congenital heart disease that has been mentioned in only one report to our knowledge. We describe the case of a 62-year-old male patient suffering from recurrent abdominal distention, nausea, and lower extremity edema. The right ventricular morphologic abnormalities as shown by echocardiography and CT were comparable to those of left ventricular apical hypoplasia, suggesting right ventricular apical hypoplasia. However, this speculative diagnosis remains to be confirmed by additional cases. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 46:82-84, 2018.